AB0832 CHRONIC INTESTINAL PSEUDO-OBSTRUCTION IN PATIENTS WITH SYSTEMIC SCLEROSIS
Authors: I. Blazquez, A. Guillen-Del-Castillo, J. Corada, C. Codina, A. Gil, J. Mestre, A. Antón, J. Perurena-Prieto, C. Malagelada, L. Alcala-Gonzalez, C. P. Simeon Aznar
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Chronic intestinal pseudo-obstruction (CIPO) is one of the gastrointestinal manifestations related to systemic sclerosis (SSc). Despite its low incidence (5%), it burdens a high morbimortality. <h3>Objectives</h3> The objective of the present study was to analyse a selected cohort of patients with SSc and CIPO and to describe the treatments they received, the response to them and the clinical outcome. <h3>Methods</h3> SSc patients diagnosed with CIPO were selected for the study. There was analysed the baseline clinical variables, the period between the diagnosis of SSc and CIPO, the received therapies, and the clinical outcome. <h3>Results</h3> All the seven cases we studied were women, with a median (interquartile range, IQR) age at SSc diagnosis of 46.2 years (34-52). All of them met the ACR/EULAR 2013 classification criteria for SSc. Five (71.4%) of the patients presented diffuse cutaneous SSc (dcSSc), while the other two had the limited cutaneous SSc (lcSSc) subset. The median period between the SSc diagnosis and CIPO was 7.1 years (1.84-15.96). Concerning basal characteristics, 4 (57.1%) patients presented cardiac involvement, 3 (42.8%) arthritis, 3 (42.8%) interstitial lung disease, and 1 (14.2%) pulmonary arterial hypertension. Regarding specific SSc autoantibodies, anti-centromere antibodies were detected in 2 (28.6%) patients, anti-U1RNP in 2 (28.6%) and anti-RNA polymerase III in 1 (14.2%). Six (85.7%) had concomitant small intestinal bacterial growth (SIBO), with positive response to courses of antibiotics. Regarding CIPO treatment, all of them received dietetic recommendations, 6 (85.7%) prokinetics (cinitapride, prucalopride or erythromycin) and 4 (57.1%) the somatostatin analogue octreotide subcutaneous (sc). At CIPO onset, 3 (42.8%) patients had serious oral intolerance, two of them required parenteral nutrition (PTN), while the other received intravenous immunoglobulins (IVIG) and percutaneous endoscopic gastrostomy. During the first year of follow-up after an adequate initial response to treatment, 5 (71.7%) patients had a relapse into CIPO symptoms. Among them, one improved after initiating octreotide sc, 3 required PTN, 2 IVIG and 1 case was treated with adalimumab. Four (57.1%) patients died during the follow-up, three of them as a consequence of gastrointestinal complications related to CIPO, and the fourth because of sudden death. <h3>Conclusion</h3> CIPO is a gastrointestinal manifestation of SSc, that despite its low prevalence, it represents a high morbi-mortality. Patients with dcSSc might have a higher susceptibility for CIPO. There is a substantial variability period between the SSc onset and the appearance of CIPO related symptoms. Diet recommendations, prokinetics and octreotide are common initial therapies, reserving IVIG and PTN for refractory cases or with more aggressive onset. Notwithstanding, the mortality related to this gastrointestinal involvement is certainly significant. <h3>References</h3> [1]Shah, J., &amp; Shahidullah, A. (2018). Chronic intestinal pseudo-obstruction in systemic sclerosis: An uncommon presentation. Case Reports in Gastroenterology, 12(2), 373–378. https://doi.org/10.1159/000490526 [2]Shreiner, A. B., Murray, C., Denton, C., &amp; Khanna, D. (2016). Gastrointestinal manifestations of systemic sclerosis. <i>Journal of Scleroderma and Related Disorders</i>, <i>1</i>(3), 247–256. https://doi.org/10.5301/jsrd.5000214 <h3>Disclosure of Interests</h3> None declared